髓过氧化物酶-抗中性粒细胞胞质抗体相关性血管炎132例临床分析  

A study on clinical characteristics of 132 patients with vasculitis associated with antineutrophil cytoplasmic antibody to myeloperoxidase

作　　者：吕远帆 帅宗文[1] 张铭明[1] 胡子盈 

机构地区：[1]安徽医科大学第一附属医院风湿免疫科,合肥230022

出　　处：《中华风湿病学杂志》2014年第5期308-312,共5页Chinese Journal of Rheumatology

基　　金：中华医学会临床医学科研专项资金项目（08010290107）

摘　　要：目的分析髓过氧化物酶（MPO）-ANCA相关性血管炎的临床特点，初步探讨MPO—ANCA与相关血管炎临床损害之间的关系。方法前瞻性研究132例细胞核周型（p）-ANCA及MPO—ACNA均阳性的初诊原发性ANCA相关性血管炎患者的临床资料，分析主要临床损害的特点，检测周围血D．ANCA和MPO．ANCA水平并探讨其与临床损害之间的关系。采用t检验和Spearman等级相关分析对资料进行统计学分析。结果来自8个不同的临床学科的132例患者中，显微镜下多血管炎（MPA）128例（97．0％），肉芽肿多血管炎（GPA）3例（2．3％），嗜酸细胞肉芽肿性血管炎（EPGA）1例（0．8％）。患者平均年龄（62±15）岁，平均确诊时间（10±18）个月，其中仅10．6％（14例）患者发病1个月得到确诊。主要临床损害中，累及肾脏72．0％（95例）、肺脏67．4％（89例）、关节26．5％（35例），心脏19．7％（26例），周围神经17．4％（23例）、皮疹10．6％（14例），中枢神经9．8％（13例）等。高龄患者更容易发病早期累及肺脏[（66±11）岁，（55±19）岁，t=-3．478，P〈0．01；（6±10）个月，（18±27）个月，t=2．920，P〈0．01]，关节累及者非高龄者为多[（57±18）岁，（64±13）岁，t=2．335，P〈0．05]。p-ANCA水平与病情活动度及脏器累及范围无关（r=0．013，P〉0．05；r=0．087，P〉0．05），MPO．ANCA水平与病情活动度正相关（r=0．258，P〈0．01），与损害脏器的范围无关（r=0．022，P〉0．05）。结论我国MPO—ANCA血管炎并不少见，以主要累及老年人群的MPA为主，多器官损害的临床表现缺少特异性，早期延误诊断者多。MPO—ANCA可能是其相关血管炎的致病抗体，临床表现的多样性可能与MPO—ANCA的不同特异性有关。tADstractl UbjecUve lo analyze the cIlmeal characteristics ot vaseulltlS assocmted with antmeu- trophil cytoplasmic antibody against myeloperoxidase （MPO-ANCA）, and to investigate preliminarily the rela- tionship between MPO-ANCA and the clinical damages. Methods One hundred and thirty-two patients with primary antineutrophil cytoplasmic antibody （ANCA） associated vasculitis, which were diagnosed for the first time, were involved into this prospective study. All the patients had positive laboratory tests for pert-nuclear ANCA （p-ANCA） and MPO-ANCA. The characteristics of their clinical presentations were analyzed. The levels of p-ANCA and MPO-ANCA in the peripheral blood were detected and the relationship between the levels and the damages were explored. T-test and Spearman rank correlation analysis were used for statistical analysis. Results Of the 132 patients from 8 different clinical departments, 128 （97.0%） were microscopic polyangiitis （MPA）, 3 （2.3%） were granulomatosis with polyangiitis （GPA）, and 1 （0.7%） was eosinophilic granulomatosis with polyangiitis （EGPA）. The mean age was （62＋15） years old. The average time between onset of the disease and diagnosis was （10-＋18） months, and only 14（10.6%） were diagnosed within one month. Among the major organ involvements, the occurrence of renal, lung, joint, heart, peripheral nerve,skin, and central nerve involvement was 72.0%（95 cases）, 67.4%（89 cases）, 26.5%（35 cases）, 19.7% （26 cases）, 17.4%（23 cases）, 10.6%（14 cases） and 9.8%（13 cases）, respectively. Lung was more suscep- tible to be involved among the aged in their early course [（66±11） years, （55-＋19） years, t=-3.478, P〈 0.01; （6±10） months, （18±27） months, t=2.920, P〈0.011, and joint involvement was more common in the younger [（57±18） years vs （64±13） years, t=2.335, P〈0.05] patients, p-ANCA had no relationship with the disease activity or the range of organ involvements（r=0.013, P〉0.05

关 键 词：抗中性粒细胞胞质抗体 抗髓过氧化物酶抗体 ANCA相关性血管炎 临床损害 致 病机制 

分 类 号：R593.2[医药卫生—内科学]